Semin Respir Crit Care Med 2003; 24(5): 499-530
DOI: 10.1055/s-2004-815601
Copyright © 2003 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Bronchiolitis Obliterans Syndrome Complicating Lung or Heart-Lung Transplantation

John A. Belperio1 , Kathleen Lake2 , Henry Tazelaar3 , Michael P. Keane1 , Robert M. Strieter1 , Joseph P. Lynch, III1
  • 1Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California
  • 2Department of Medicine, Division of Nephrology, University of Michigan Medical Center, Ann Arbor, Michigan
  • 3Department of Pathology, Mayo Clinic, Rochester, Minnesota
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Publication History

Publication Date:
15 January 2004 (online)

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ABSTRACT

Lung transplantation is a therapeutic option for patients with end stage lung diseases, but long-term survival remains poor, primarily due to chronic allograft rejection. Bronchiolitis obliterans (BO), a fibrotic process resulting in progressive narrowing of bronchiolar lumens and airflow obstruction, is a manifestation of chronic allograft rejection. The term obliterative bronchiolitis (OB) is synonymous. Once bronchiolitis obliterans syndrome (BOS) develops, progressive decline in pulmonary function is typical; most patients die of respiratory failure within 5 years of onset. The diagnosis of BOS is usually made by clinical, physiological, and radiographic parameters. The dominant risk factor for BOS is acute allograft rejection, but additional factors play contributory roles [e.g., infections; human leukocyte antigen (HLA) mismatching; and injury to the allograft or airways]. The pathogenesis of BOS is complex and involves myriad cell types (both immune and nonimmune) and release of diverse cytokines and chemokines. Unfortunately, current therapies for BOS are of unproven value. A greater understanding of the pathogenic mechanisms operative in BOS are critical to developing novel strategies to treat and prevent this devastating complication.

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